What is the consequence of the defect in glucose-6-phosphatase in Von Gierke's disease?

In Von Gierke's disease, a deficiency in glucose-6-phosphatase leads to an inability to effectively convert glucose-6-phosphate into free glucose, which is necessary for maintaining normal blood glucose levels during fasting or between meals. Under normal circumstances, glucose-6-phosphatase is essential in the liver and kidneys for producing glucose from glycogen and other precursors.

When this enzyme is defective, the liver cannot release glucose into the bloodstream. As a result, individuals with Von Gierke's disease experience long periods of low blood glucose levels (hypoglycemia) because the stored glycogen can only be partially broken down into glucose-6-phosphate, which cannot be converted to glucose due to the enzyme deficiency. Consequently, the body is unable to utilize glycogen effectively to maintain stable glucose levels, especially during fasting.

Other options do not accurately describe the consequences of this enzymatic defect. High blood glucose between meals would not occur since glucose release is impaired. Excess glycogen storage may occur, but it is not the main focus of the consequences related to blood sugar levels. Similarly, decreased synthesis of glucose does not directly capture the impact of the enzyme defect, as the issue lies more in the release and utilization rather